KEY POINTS

Bladder exstrophy is a severe abnormality of the anterior abdominal wall in which the bladder protrudes under the umbilical cord, the pubic bones are separate causing rectus divergence, and frequently is associated with inguinal hernia.

In females the mons, clitoris, and labia are separated, with hemiclitoris on either side of the bladder, and there is frequently duplication of the vagina and uterus.

In males the penis is short and broad, with a dorsal urethral plate, dorsal chordee, and splayed glans.

Bladder exstrophy is very rare, being seen in 1 in 3000 to 1 in 50,000 births, and there is a significant male preponderance.

Prenatal sonographic diagnosis is possible by noting an absent bladder, but with normal amniotic fluid volume, together with a lower midline abdominal wall mass and abnormal appearing external genitalia.

Pregnancy management is generally unchanged by the prenatal diagnosis of bladder exstrophy, and the only role for genetic amniocentesis is to assist in assignment of gender if external genitalia are unclear.

The goal of surgical management is to achieve bladder closure, epispadias repair, and achievement of urinary continence; this can be achieved either in a three-stage process over 4 years or as a single primary repair.

Bladder exstrophy is generally considered a sporadic abnormality, although there may be a 3%–4% recurrence risk in siblings.

CONDITION

Exstrophy of the bladder has been recognized for centuries, but it was not until the 19th century that surgical correction was first attempted (Hall et al., 1953). The first attempts at diversion of urine into the colon were made in 1850, and the first successful closure of bladder exstrophy was performed in 1862 (Canning et al., 1996). In contrast to patients with cloacal exstrophy that have other unrelated abnormalities, infants with bladder exstrophy have defects confined to the bladder, abdominal wall, perineum, genitalia, and bony pelvis.

At birth the diagnosis of bladder exstrophy is easily made by the presence of characteristic findings. The bladder plate protrudes immediately beneath the umbilical cord (Figure 65-1). The rectus muscles are divergent due to separated pubic bones. There is an outward rotation of the innominate bones and eversion of the pubic rami (Sponsellor et al., 1991). The phallus is short, with a dorsal urethral plate, splayed glans, and dorsal chordee. In females, the mons pubis, clitoris, and labia are separated and the vaginal orifice may be displaced anteriorly. Bilateral inguinal hernias are commonly seen at birth because of the large internal and external inguinal ring caused by the splaying of the rectus musculature and lack of obliquity of the inguinal canal. Hussman et al., (1990) reported that 56% of males and 15% of females have inguinal hernias. In a report by Peppas et al., (1995) in patients presenting with hernia within one year of primary closure, 10%–53% of the hernias were incarcerated.